Ask the PBC doctor
DISCLAIMER: The following is intended for general information purposes only. It is not intended to be comprehensive. It does not in any way constitute legal or other professional advice, and should not be relied upon as such. The reader is cautioned to consult their own physician and other experts for advice regarding specific health concerns. The Canadian PBC Society is not responsible for the accuracy, completeness, or any action taken on the basis of the information mentioned herein. It is not intended to substitute for and/or supersede one's own physician's advice.
Herbal remedies that can be toxic (PDF)
Compiled by the Liver Clinic at Toronto Western Hospital.
I was diagnosed with PBC approximately six years ago, but have had symptoms going back 30 years. I am current 50 years of age. According to my physicians, my blood work liver function tests keep coming back perfect (normal bilirubin, Alk phos, etc.) but the fatigue and energy level that I am experiencing is almost debilitating. I am on Ritalin in order to be alert at work, though the effect seems to be waning as the fatigue increases. I really care about nothing anymore — I just want to curl up and sleep all of the time. I just want to be left alone, as nobody understands that I am sick. Does the fatigue extent increase with the stage of the disease, and even though the blood work returns normal, is the disease progressing? I don’t remember what stage it was for my biopsy; either Stage 1/2 or Stage 2/3 though my understanding of this is that it is not necessarily an accurate measurement when doing needle core biopsies. [ANSWER]
Your fatigue may be still related to your PBC. I don’t think Ritalin is advisable. It is also possible that you are depressed on top of your fatigue induced by the PBC. I suggest you discuss this possibility with your family physician.
— Dr. Jenny Heathcote, 2013
Which GERD med is least likely to cause negative interaction with Ursodiol? [ANSWER]
They are all safe.
— Dr. Jenny Heathcote, 2013
I was diagnosed with PBC recently. My doctor has suggested a liver biopsy. I am really scared and worried about it. I wonder whether it is necessary and what is the purpose? Thanks a lot. [ANSWER]
We don’t often do a liver biopsy to make a diagnosis if all the blood test results suggest PBC, for example, an increase in your liver enzymes, particularly alkaline phosphatase (ALP) and positive antimitochondrial antibodies (AMA), with or without increase in IgM. You must also have a good abdominal ultrasound to look at the liver. If you also have thyroid disease and/or rheumatoid arthritis the diagnosis of PBC is more likely.
— Dr. Jenny Heathcote, 2013
Is it safe to take Green Coffee Bean extract if you have PBC? [ANSWER]
I have no idea what this is but I do advise my patients not to use alternative medicines. Unless the medication has been tested scientifically it cannot be shown to be either beneficial or harmless.
— Dr. Jenny Heathcote, 2013
I am having a really hard time losing weight. I am almost 44, female, and was diagnosed with PBC a few years ago. I take ursidol twice daily and lead a fairly active lifestyle. I am a non-smoker and seldom drink alcohol. I am 5’8” and currently weigh 178 lbs. Can I do a keto diet? Some research on the internet has led me to think maybe a diet without gluten/wheat might help in many ways. [ANSWER]
Treatment with ursodeoxycholic acid is associated with a small weight gain, rarely more than five pounds. Most often weight gain is promoted by drinking soda (diet or otherwise), drinking fruit juice or to eating fried food or carbohydrates in excess. The higher the fiber content of your food the better e.g. green leafy vegetable. No bagels or buns, only dark brown bread and no more than two pieces of fruit per day. Unless you have celiac disease proven a gluten/wheat free diet is of no value. A keto diet is dangerous!
— Dr. Jenny Heathcote, 2013
I was diagnosed with PBC approximately four years ago. My liver function tests appear to be good, but recently my creatinine is elevated. Currently I am undergoing blood work and urinalysis for diabetes. Is there a link between diabetes and PBC? [ANSWER]
No. Diabetes affects ten per cent of Canadians and is most common in those who are overweight — but is not restricted to those who are. Very, very occasionally renal disease is found in subjects with PBC but may not be a true association.
— Dr. Jenny Heathcote, 2013
I was diagnosed with PBC a year ago. Lately I find I sleep a lot in the day as well as at night. Will this continue? [ANSWER]
Fatigue occurs in up to 80% of patients with primary biliary cirrhosis. However, on diagnosis up to half of the patients that we see in clinic have no symptoms because they are generally picked up on routine screening with abnormal liver tests. Patients with fatigue often have to sleep during the day or find they have little energy to do much in the evenings. This symptom is usually persistent, although some patients do occasionally respond to medications in drug studies that appear to perk them up. However, this response only occurs in a handful of patients and therefore there are no specific drugs associated with the treatment of fatigue in patients with PBC.
— Dr. Andrew Mason, 2011
I was diagnosed with PBC in 1995 and take prescribed URSO 250 mg, 5tablets per day. I also ADEKs vitamin supplements of A,D,E, and K. In the past 6 months, I have started using Women's One A Day for those aged 50 + in conjunction with ADEKs. I understand from my pharmacist that ADEKs will no longer be manufactured. My question is this: could you please recommend what in your opinion would be an appropriate course of supplements for me to be taking to ensure optimum benefit for someone of my age - almost 56? Does the current research support any particular supplementation for PBC patients? [ANSWER]
Using women's One-a-Day for those aged greater than 50 should be sufficient for most patients with PBC. There is no proven benefit of using ADEK, and this prescription was made specifically for PBC patients without any evidence that it provided benefit. We usually recommend that patients take an age-appropriate multivitamin, as well as supplements to prevent thinning of bones. This includes vitamin D 1,000-2,000 i.u. a day, as well as calcium supplements. Previously, we used to recommend 1 to 1.5 grams of calcium citrate to prevent boney disease. However, the use of calcium supplements has been questioned recently because a study from New Zealand reported that this may be associated with the development of calcium deposits in arteries. This in turn may be associated with a slightly increased risk of developing heart attacks or other vascular diseases such as stroke. While we do not know for sure what the optimal dose of calcium is now for patients, I think it is probably wise to take some calcium supplementation to avoid the risk of thinning of bones and osteoporosis of 500mg per day.
— Dr. Andrew Mason, 2011
I was found to have PBC 18 months ago. With your estimates of cases in North America of 1:50,000, and I'm in the 10% group of PBC (male), I feel really special (1:500,000)! The only things that are in my background is a case of Hepatitis A in 1978 and treatment for ADHD with methlyner since 2000. Both of these things relate to the liver in very small ways — could either be considered a catalyst? [ANSWER]
It is unlikely that either hepatitis A or the treatment for ADHD with Ritalin are triggers for PBC. Both environmental factors are quite common, whereas PBC is rare. Therefore, it is unlikely that either of these could be considered a catalyst for PBC.
— Dr. Andrew Mason, 2011
How does PBC affect my body in combination with my other auto immune diseases such as Graves, Diabetes, Arthritis and Ulcerative Colitis,? Which specialist could best help me to address the problem of these conditions plus the medications that have to be taken which might impact PBC? [ANSWER]
Each autoimmune disorder described affects the body in different ways. The main problem with having PBC and liver disease is that it affects the absorption of fat-soluble vitamins, and therefore patients with PBC are more likely to develop thinning of the bones as they do not absorb the calcium and vitamin D. PBC may also effect energy levels and cause itching and dry eyes with dry mouth. The other diseases such as Graves' and diabetes may have some affect on heart function, and patients with diabetes can get blood vessel disease as well as kidney disease if their control of sugar is not good. Patients with ulcerative colitis may get disease of the skin with rashes, as well as arthritis. Generally speaking, if you have a specific disease that is difficult to manage, it is always worth seeing a specialist associated with that disease such as a rheumatologist for arthritis, and endocrinologist for diabetes and Graves' disease, a liver specialist for PBC, or a gastroenterologist for ulcerative colitis. However, if the diseases are well controlled, it may be simpler and more coordinated to see one physician who is familiar but not an expert in all these diseases.
— Dr. Andrew Mason, 2011
A friend with liver damage from Lyme's Disease benefitted greatly from a 'liver support' powder called 'Huan Fat Metaboliser that contained Choline, Methionine and Inositol, which, apparently, work to break down fatty proteins. Do you think that this product would be useful for PBC sufferers? [ANSWER]
It sounds like Huan fat metabolizer that contains choline methionine and inositol may possibly be a good treatment for patients with fatty liver but there are no studies that I know of that show this. Indeed, the benefit from fatty liver disease from this supplement is questionable, but there are some theoretical data to suggest that choline methionine may be of benefit for patients with fatty liver. I am unsure how this product would be useful for patients with PBC unless they were overweight and suffer from fatty liver as well.
— Dr. Andrew Mason, 2011
Is there spontaneous remission and negative AMA serology of prior positive and abnormal LFT patients. If so, how often; and is it seen with urso treatment? [ANSWER]
AMA may come and go — and may go for good with UDCA. No spontaneous remission reported.
—Dr. Jenny Heathcote, 2006
After a biopsy confirming diagnosis of PBC, what criteria is used for assessing the necessity of repeat biopsy? [ANSWER]
1) Determine if there is apically progressive bile duct loss (rising ALP) or another superimposed liver problem eg: fat
2) Evaluate progression on UDCA (may occur even if liver blood tests are normal)
—Dr. Jenny Heathcote, 2006
If a patient is on urso with stable blood results, does this make a difference in the length of time between biopsies? Does it differ with asymptomatic patients? [ANSWER]
Repeat biopsies are not performed on everyone — it really when it is done depends on the indication
—Dr. Jenny Heathcote, 2006
I have heard there is a blood test that is just as accurate as a liver biopsy to determine the stage of PBC. Is this true? [ANSWER]
True — what is truth? Some may think there are reliable blood tests (eg. Dr. Poynard in Paris) — but some of us are not convinced yet!
—Dr. Jenny Heathcote, 2006
Have you heard or seen adverse reactions to urso, characterized by widespread thickened skin eruption? [ANSWER]
No. In our initial randomized controlled trial two patients dropped out because of a skin rash (turned out both were on placebo!)
—Dr. Jenny Heathcote, 2006
If a patient with high cholesterol, given a statin (lipitor), develops high AST/ALT, is this considered a class drug reaction, or could they possibly change to another statin- e.g. crestor, with safety? [ANSWER]
Most abnormal liver tests in those on statin are due to fat in the liver — not the drug — not much to worry about.
—Dr. Jenny Heathcote, 2006
Is there any slow release or long-acting form of urso being considered for the future? [ANSWER]
No
—Dr. Jenny Heathcote, 2006
For someone with a family history of stroke and/or heart attacks is there a concern with taking 81mg of aspirin daily? [ANSWER]
Not unless they have esophageal varices and/or ascites.
—Dr. Jenny Heathcote, 2006
What suggestions do you have for a woman with PBC going through severe menopausal symptoms such as: hot flashes, sleeplessness and mood swings. [ANSWER]
HRT is safe but may increase bilirubin, which returns to normal off HRT.
—Dr. Jenny Heathcote, 2006
I notice many symptoms get added as enough people mention them. Is there any advice for those of us with very weak and painful hands (particularly) and other joint and muscle pain if RA has been ruled out? [ANSWER]
Tylenol is quite safe if taken as recommended.
—Dr. Jenny Heathcote, 2006
Is swimming in a chlorinated pool OK? [ANSWER]
Yes.
—Dr. Jenny Heathcote, 2006
With the threat of osteoporosis, what amount of supplemental calcium (and in what form) do you recommend if at all for patients who are 50+? [ANSWER]
1500 mg elemental Ca/d + Vitamin D 800-1000iu/d.
—Dr. Jenny Heathcote, 2006
I was at a liver foundation talk by a dietician and she informed us that we should be on a high protein diet. This went against my understanding. Please advise? [ANSWER]
Standard amounts of protein are fine — no need for high or low protein diet.
—Dr. Jenny Heathcote, 2006
Are there medications that are safe for HBP? [ANSWER]
HBP=High Blood Pressure — all safe
—Dr. Jenny Heathcote, 2006
Are there any Irritable Bowel type symptoms associated with PBC? [ANSWER]
No.
—Dr. Jenny Heathcote, 2006
I have read that one does not get PBC without some other so-called auto-immune disease as well. Has this been found to be true? [ANSWER]
No. Other autoimmune diseases are common but not the rule.
—Dr. Jenny Heathcote, 2006
Should PBC patients be getting the Twinrix vaccination? [ANSWER]
Always a good idea.
—Dr. Jenny Heathcote, 2006
Is it safe to take Flexeril with URSO? [ANSWER]
Probably.
—Dr. Jenny Heathcote, 2006
Is there a need to phase off cholestyramine or can you just stop taking it? [ANSWER]
Just stop; “cold turkey” is safer.
—Dr. Jenny Heathcote, 2006
Should one get a flu shot? I heard some get the flu after receiving the vaccination- is this true? [ANSWER]
You may, but more likely a good idea.
—Dr. Jenny Heathcote, 2006
Is it safe to take milk of magnesia (60ml) approx 3-4 times per month for constipation? [ANSWER]
Yes.
—Dr. Jenny Heathcote, 2006
What non-prescription cold medicine is least harmful to the liver? [ANSWER]
Most over-the-counter cold medications are reasonably safe with liver disease. The main issue is the sedating aspect of these drugs particularly in patients with advanced liver disease. Patients should ask their pharmacist for the least sedating cold medication.
Acetaminophen is actually the safest pain medication to take in patients with liver disease as long as the total dose per day is less than 4 gms. [ANSWER]
—Dr. Nigel Girgrah, 2006
How does Echinacea affect the liver? [ANSWER]
There are case reports of Echinacea leading to liver inflammation and very rarely to acute liver failure. Also, Echinacea may interact with other medications and may affect the way certain drugs are broken down by the liver. Therefore, I think it is safest to avoid Echinacea if you have liver disease.
—Dr. Nigel Girgrah, 2006
How does Milk Thistle affect the liver? [ANSWER]
Milk thistle, I think is reasonably safe, in patients with liver disease. Whether it helps patients with liver disease is debatable, but there is a small number of studies that show clear benefits. Patients should also be aware that it is quite expensive and should factor this into their decision-making when thinking about taking Milk Thistle.
—Dr. Nigel Girgrah, 2006
Can pruritis be caused by too high a dosage of URSO? [ANSWER]
Some patients may experience worsening pruritus on URSO. However, I am not aware that is related to the amount of URSO taken.
—Dr. Nigel Girgrah, 2006
Would it be safe for a PBC patient to take Tamiflu? [ANSWER]
As far as I know, Tamiflu is safe to take if you have PBC.
—Dr. Nigel Girgrah, 2006
Is nausea a common symptom of PBC and can anything be done about it? [ANSWER]
Nausea is not a common symptom of PBC. Nausea is usually associated with intestinal problems and a common side effect of taking medications. Fortunately, both Ursodiol and Cholestyramine seldom cause nausea and both drugs are generally well-tolerated. Other medications may cause nausea, especially on an empty stomach. For example, multivitamins can cause a degree of nausea if not taken with food. Common intestinal diseases associated with nausea include peptic ulcer disease, irritable bowel disease as well as other inflammatory bowel conditions. If nausea is a significant problem, it should be discussed further with your doctor.
—Dr. Andy Mason, 2006
Can we take vitamin B3? Is Niacin and Niacinamide the same thing or if different, is one safer? [ANSWER]
Niacin is a water soluble vitamin also known vitamin B3. There are different formulations including Nicotinic Acid and Nicotinamide. Nicotinamide is generally better tolerated than Nicotinic Acid and does not generally cause blushing, however nausea, vomiting and signs of liver toxicity may occur when high doses of the this supplement are taken. This may be observed with doses of 3 g/day. The current recommendation is to take about 20 mg/day. Patients usually receive enough Niacin in meats, poultry, fish, cereals, vegetables and seeds. Milk also provides Niacin as well. Niacin used to be used to treat high cholesterol but due to liver toxicity, newer medications are preferred such as the "Statins". Personally, I do not recommend additional vitamin B3 for patients more than can be found in standard multivitamin preparations. Accordingly, I advise most patients with PBC to take a multivitamin with extra vitamin D, 1000 IU/day as well as 1 to 1.5 g of calcium to avoid bone disease.
—Dr. Andy Mason, 2006
Do you recommend vitamin B12 shots for fatigue for PBC if it's safe. [ANSWER]
Vitamin B12 shots are safe and no toxicity or adverse affects have been associated with large doses. Vitamin B12 deficiency is estimated to affect 10 to 15% of individuals over the age of 60 and is specifically associated with pernicious anemia, which is also an autoimmune condition. Deficiency is also seen with vitamin B12 malabsorption. Both these conditions are distinct from primary biliary cirrhosis and therefore I do not generally recommend vitamin B12 shots for fatigue for PBC. However, up to 5% of patients with PBC can develop the malabsorption syndrome celiac disease that can be associated with low vitamin B12 levels. I would consider evaluating the levels of vitamin 12 in patients with (i) low hemoglobin, (ii) antibodies to intrinsic factor which helps with vitamin B12 absorption, (iii) signs of pernicious anemia and vitamin B12 deficiency with sore tongue, tingling in arms and legs and other neurological symptoms. If the vitamin B12 level is low - I would recommend the use of B12 shots. As part of your work-up for PBC, your doctor may arrange a gastroscopy, look for signs of inflammation in the stomach and may also check other autoantibodies associated with autoimmune conditions that are linked with vitamin B12 loss.
—Dr. Andy Mason, 2006
Do problems with clotting start in the early stages of PBC? [ANSWER]
It is unusual for clotting problems to be present in the early stages of PBC unless it is linked with another disease where clotting is a problem. Clotting problems generally start when patients have cirrhosis, which is associated with low platelets as well as a diminished production of clotting factors from the liver. However, sometimes patients with primary biliary cirrhosis can have problems with absorbing their fat-soluble vitamins - A, D, E and K. Vitamin K deficiency is associated with lack of production of clotting factors. So if patients have a lack of vitamin K early on in their disease, they could theoretically develop clotting problems. Once again, I recommend a multivitamin for all patients with primary biliary cirrhosis to avoid these problems.
—Dr. Andy Mason, 2006
Is hepatic encephalopathy of PBC corrected or improved following successful liver transplant? [ANSWER]
Yes, in all cases. However, there are other things to consider as well. Patients may have problems with concentrating or thinking due to conditions unrelated to their liver disease and PBC. If this is the case, a degree of neurological disease may continue following transplantation. Also, following liver transplantation some of the drugs used for immunosuppression can make patients concentration a little fuzzy.
—Dr. Andy Mason, 2006
Why do patients experience aching joints? [ANSWER]
Primary biliary cirrhosis, and autoimmune diseases in general, have several features in common. For example, patients with primary biliary cirrhosis may experience symptoms that patients with other rheumatological diseases experience as well. Patients with primary biliary cirrhosis and Sjogren’s syndrome experience dry eyes and dry mouth. Patients with primary biliary cirrhosis also experience aching joints and aching muscles that patients with rheumatological diseases experience. In fact, this is not uncommon in patients with other forms of liver disease such as hepatitis C virus infection, hepatitis B virus infection and hemochromatosis (an iron storage disease) who will also experience aching joints. As long as the pain is not too severe and the joints are not swollen or deformed, we usually recommend taking Tylenol in regular doses to treat aching joints. If the joint problems are severe, we often look for other courses, such as rheumatological disease.
—Dr. Andy Mason, 2006
Should children of PBC patients have their AMA checked? [ANSWER]
We know that family members of patients with PBC have a 10 to 15-fold increased risk of developing the disease. However, we have to take into account that PBC is a rare disease. In North America, PBC occurs 1 in 10,000 to 1 in 50,000 people. This means an increased risk of tenfold translates to family members having a 1:1000 to 1:5000 risk of PBC. I usually tell patients that children do have increased risk and if they have increased liver function tests when they have their general physicals done, they should also have an AMA checked if their liver tests are abnormal. We also recommend having an AMA checked if relatives of patients start to experience signs and symptoms of primary biliary cirrhosis such as fatigue, pain in the right side of the abdomen, dry eyes, dry mouth or itching. Otherwise we do not recommend routine AMA checking.
—Dr. Andy Mason, 2006
What research is currently being done with PBC? [ANSWER]
There are several studies in PBC, worldwide. There are a couple of studies in Canada that are noteworthy. At the University of Alberta, we have just completed an international multi-center randomized controlled trial looking at how combination anti-viral treatment with Combivir impacts on primary biliary cirrhosis. We found a significant decrease in liver function tests in patients on Ursodiol therapy who received the Combivir treatment rather than the placebo. The trial was a proof of principals study to determine whether anti-viral therapy actually worked for PBC. Although the results were significant, the effects were not substantial. We are therefore working on a mouse model of PBC to determine better combinations of anti-viral therapy that will be both well-tolerated and effective in halting disease.
—Dr. Andy Mason, 2006
In other studies, Dr. Jenny Heathcote and Kathy Simonovitch are conducting a PBC family study looking at the genes that may be associated with the development of PBC. They plan to enroll ~ 1000 patients with PBC as well as relatives if possible. For this study they will compare the gene of patients with PBC and control subjects to see if there are some differences that are found more often in patients with PBC. These studies have the potential to find disease-causing genes that may lead to better treatment strategies for PBC. In combination we hope that we will find anti-viral treatments and new therapies to combat the genes that may predispose to PBC. [ANSWER]
—Dr. Andy Mason, 2006
Do cholesterol drugs affect liver function tests? [ANSWER]
Cholesterol drugs are often required in patients with PBC. This is because when bile ducts are blocked, the cholesterol cannot get out of the liver and it tends to build up in the body. We know that cholesterol can lead to problems with coronary artery disease and therefore we do recommend anti-cholesterol therapy for patients that are at risk for coronary artery disease, heart attacks and strokes. The drugs that are referred to as “Statins”, such as Lipitor, are commonly used in the population to combat cholesterol. These medications are very safe. On occasion, they can cause an increase in liver function tests. However, as patients with PBC are monitored regularly for their liver function tests, it is usually quite safe for them to take these medications. If their liver tests become worse on the Statin, then the dosage can be modified or the drug can be discontinued. There are also some data which suggest that other lipid (fat) lowering agents such as the fenofibrates also positively impact on the progression of primary biliary cirrhosis. This has not been tested in a clinical controlled trial, however. These drugs are both safe and can potentially improve PBC. The difference between the fenofibrates and the statins is that the fenofibrates can also reduce other fats such as the triglycerides as well as the cholesterol in the body.
—Dr. Andy Mason, 2006
What causes brain fog with PBC? [ANSWER]
Dr. Mark Swain at the University of Calgary works on the neurological changes associated with cholestatic disease. There are several complicated mechanisms involved with the brain fog and fatigue associated with PBC. We understand now that, like other liver diseases including hepatitis C virus infection, the problem is not just isolated to the liver. Indeed, some patients with near-normal liver function tests can have quite severe fatigue and brain fog as well. It is currently not known what causes this. On occasion, patients may benefit from some forms of anti-depressant therapy to pick them up. However, these are not recommended unless patients are very incapacitated from their liver disease and cannot function.
—Dr. Andy Mason, 2006
Is unsteadiness on the feet a common occurrence with PBC and, if so, why does it occur? [ANSWER]
The group in Newcastle in the United Kingdom is currently studying this. It appears that some patients with PBC have swings in their blood pressure. Sometimes their blood pressure is high and sometimes their blood pressure is low. This means that when you stand up too quickly, the blood supply to the head can be reduced and patients can become unsteady on their feet and dizzy. Again, it is not known what causes this change in the nervous system controlling the blood pressure; and this is subject to research. Unsteadiness on the feet may occur for several other reasons, and if this is becoming a problem, then patients should see their doctor to be worked up for other causes of unsteadiness that include neurological or potentially cardiac causes or disease.
—Dr. Andy Mason, 2006
Is there a safe cholesterol medication to take? [ANSWER]
Most cholesterol medications are safe for people with PBC. The statins, such as Lipitor, rarely cause any changes in liver function tests. As patients with PBC have their liver function tests done regularly anyway, we can usually spot when patients are having a reaction to this treatment. Also, cholesterol medications such as fenofibrates such as Tricor may also be of benefit to reduce cholesterol and triglycerides in patients with PBC. The only drug that reduces cholesterol that may be bad for PBC patients is niacin, which causes a dose-dependant damage to the liver. Patients who have high cholesterol and other risk factors for heart disease such as smoking, hypertension, diabetes, or a family history of heart attacks at a young age could be considered patients for cholesterol treatment.
—Dr. Andy Mason, 2008
What is the risk for us getting or not getting hepatitis shots? [ANSWER]
Patients who travel to tropical countries may be exposed to hepatitis A. Patients that have sexual or exposure to blood products from patients with hepatitis B may be at risk of getting hepatitis B. It is thought that patients who already have liver disease may have worse reactions if they get hepatitis A or B. So we would recommend patients getting shots for hepatitis B and hepatitis A if travelling.
—Dr. Andy Mason, 2008
How does the AMA affect the liver? And other body structures? [ANSWER]
AMA is a diagnostic marker for PBC. Some patients with PBC, however, have no AMA in their blood. This is a small percentage of approximately 1 in 20 patients with PBC. It is unknown whether AMA does affect the liver or whether this is just a marker for disease. As patients with no AMA may get PBC, I suspect that AMA is not the cause of the liver disease, but a marker of PBC.
—Dr. Andy Mason, 2008
At what T score level should a PBC patient with a low Bone Density consider a bisphosphonate a necessity? [ANSWER]
This is not a straightforward question. All patients should be on calcium and vitamin D over 1000 i.u. a day. to prevent bone loss. If people have a low bone density despite taking vitamin D and calcium, then a bisphosphonate treatment can be considered. We could either use the drug such as Didronal to inhibit the breakdown of bone, or the stronger bisphosphonates such as Fosamax.
—Dr. Andy Mason, 2008
What are the chances that increasing by 50% to a max Calcium (1000mg0 and Vit D (500mg) daily supplement will slow further resorption AND increase bone mass without the addition of a bisphosphonate for the average senior PBC patient? [ANSWER]
In the first instance I would recommend increasing the calcium and vitamin D to calcium 1.5 grams per day and vitamin D 1000 i.u. per day. Following this, only monitoring with bone density would be able to determine whether this is effective in building up the amount of bone in a patient. Individuals vary, and therefore this assessment needs to be done on a patient to patient basis. However, if patients remain with low T scores within range for fracture despite increased calcium and vitamin D, I would certainly recommend a bisphosphonate.
—Dr. Andy Mason, 2008
Is it safe for a PBC patient to be pregnant? Should she continue to take Urso? [ANSWER]
In general, yes. If they have advanced liver disease then it may be very difficult to get pregnant and if they do, there may be increased problems. Stopping Urso because of pregnancy is controversial. in animal studies Urso does not appear to be teratogenic, but the manufacturer does not recommend using it in pregnancy. It is really the 1st trimester which is of concern, and Urso is widely used to treat cholestasis of pregnancy safely. I recommend that my patients stop Urso when trying to get pregnant and they can start again in the 2nd trimester.
—Dr. Mark G. Swain, 2007
Is there a relation of rectal bleeding to PBC? [ANSWER]
No, unless there are hemorrhoids which may rarely be related to liver disease.
—Dr. Mark G. Swain, 2007
Is there a link between pruritus and restless leg syndrome and PBC? Is there a diet or supplementary to alleviate this?[ANSWER]
Not directly, but certainly pruritus may make it worse. Not that I am aware of.
—Dr. Mark G. Swain, 2007
Are telangiectasias related to PBC? [ANSWER]
Typical telangiectasias can be related to cirrhosis, but are also common in people without liver disease.
—Dr. Mark G. Swain, 2007
Are asthma medications harmful to the liver? Namely, Alvesco and/or Advair. [ANSWER]
No.
—Dr. Mark G. Swain, 2007
Is there a higher incidence of kidney stones among PBC patients? [ANSWER]
I am not aware of any published reports on an increased frequency of kidney stones in PBC patients. However, theoretically, PBC patients do have a higher incidence of something called distal renal tubular acidosis (a kidney acid-base defect) and this has been associated with an increase in renal stone formation.
—Dr. Mark G. Swain, 2008
Is there any difference between the Axcan URSO and the new generic drugs? What about the non-active ingredients? ? [ANSWER]
In general, the non-active ingredients are different and this may theoretically lead to altered pharmacokinetics (ie. rate of absorption and plasma levels) of the 2 URSO compounds. In addition, I have noted that some patients may experience intolerance to some of the non-active ingredients (eg. rash, diarrhea). However, in general, given the cost differential I typically support a trial of the generic URSO and if it is effective biochemically (ie. the liver tests fall in newly started patients, or do not rise after switching patients currently taking Axcan URSO to an equivalent dose of generic URSO) and does not cause any adverse side effects I continue on with the generic form.
—Dr. Mark G. Swain, 2008
How can one pace daily activities and preserve stamina and energy when carrying out a full time job and not feel exhausted at the end of ones day? Will it make the disease progress faster working full time and being fatigued all the time? [ANSWER]
Very good question and an important issue for patients with PBC and who experience fatigue. Given that the cause of fatigue in PBC is unknown specific recommendations are not possible. However, typically eating reasonably and keeping ones weight under control, trying to rest when the situation allows and getting a good nights sleep, and keeping as aerobically fit as possible, are mainstays in the approach to fatigue. Try to take a nap when you first get home at the end of the day or you could even try meditation. Make sure that your doctor has ruled out other common causes of fatigue that are specifically treatable (eg. anemia, renal impairment, thyroid disease, diabetes). Sometimes, a full-time job is not tenable and a modification of your work schedule may be needed (ie. part time, working some from home).
Fatigue actually does not correlate with severity of disease in PBC patients in general, and there is no evidence that working while being fatigued impacts on the progression of the liver disease.
—Dr. Mark G. Swain, 2008
I have heard it is bad for the liver to go for extended periods without food. Could you explain this? [ANSWER]
I guess it depends upon how long of a period one is going without food. In general, extended periods of food deprivation will not hurt your liver.
—Dr. Mark G. Swain, 2008
Have you heard of Lyrica and Cymbalta. I know some American PBCers are on these drugs for pain. What is your take on them.? [ANSWER]
Lyrica is an anti-seizure medication and Cymbalta is an anti-depressant. However, both of these drugs have been approved in the USA to treat neuropathic pain (ie. pain coming from diseases which affect or damage nerves; such as diabetes). In addition, Lyrica can be used for the treatment of the pain associated with fibromyalgia. In general, these are "relatively safe" drugs and could be used in the setting of PBC (after discussion with your family doctor) if the individual was suffering from one of these conditions associated with pain and they had no other contraindications to their use.
—Dr. Mark G. Swain, 2008
My wife has had PBC for over 10 years now and in the last 2 years she has had kidney stones.Her urologist first suggested that she stop taking the Urso medication as he thought that this was contributing to the kidney stones. Because she has to take the Urso to slow the progress of the PBC she could not stop taking it and he put her on a lime juice diet. My question is: Do you know of a relation between kidney stones and the Urso medication? [ANSWER]
I am not aware of any association between Urso and kidney stones. I would not recommend that your wife stop the Urso because of kidney stones.
—Dr. Mark G. Swain, 2009
Has there been any indication that nail polish may either increase your chances of developing PBC or of causing PBC? [ANSWER]
This is a very interesting and controversial question. In a patient interview study from Dr. Eric Gershwin's group in California (published in Hepatology, 2005), exploring potential lifestyle risks for PBC, they identified frequent use of nail polish as slightly increasing the risk for the development of PBC. Importantly, this increase was very slight.
—Dr. Mark G. Swain, 2009
How would the itching related to PBC be described in layman's terms by a patient? I am experiencing itchy burning and tingling inside my body, particularly on my upper arms, one arm being worse than the other, which has been leaving bruising from scratching in the night. [ANSWER]
Itching in PBC is truly an individual experience. What you describe is not uncommon - a burning, tingling sensation in the skin. The itching associated with PBC is often felt on the soles of the feet and palms of the hands, but may be experienced on any part of the body. Everyone's experience differs. If you are itching to the point of scratching at night to cause bruising you should contact your treating physician to explore anti-itch therapies which can often really help.
—Dr. Mark G. Swain, 2009
Can Urso lead to stomach problems - such as irritation ,pain, cramps etc. [ANSWER]
Yes. Urso is a bile acid and as such can cause stomach irritation. Taking it with food or at night before bedtime can often improve this.
—Dr. Mark G. Swain, 2009
Are there any cases of reversal of liver damage that are documented? [ANSWER]
The reversal of liver scarring in PBC by therapy is controversial. Dr. Marshall Kaplan in Boston has described patients in the literature who appear to have had remarkable reversals of liver scarring when treated with Urso plus methotrexate. However, these findings are not broadly accepted and in general the liver scarring which occurs in PBC should be viewed as irreversible.
—Dr. Mark G. Swain, 2009
Have there been any studies on the relationship between nutrition and liver health (in order to maximize liver function in the PBC patient)? [ANSWER]
In general PBC patients should follow a healthy, well balanced diet. If a patient is from European descent then testing for celiac disease would be reasonable (will be present in roughly 6% of PBC patients). If a patient is overweight, then losing weight through a well balanced diet and weight bearing exercise, to maintain a normal or near normal BMI, would be reasonable.
—Dr. Mark G. Swain, 2009
Is diabetes more common in patient's with PBC? [ANSWER]
PBC is an autoimmune disease and certain other autoimmune diseases are often seen in association with it. Early onset, type 1, diabetes is however not one of those frequently seen - the common ones seem to be hypothyroidism, Sjogrens, scleroderma and rheumatoid arthritis. Late onset diabetes is very common generally, but is not obviously more common in those with PBC than in the general population.
—Dr. Gideon Hirschfield, 2008
Are you more prone to esophageal bleeds once you have had one? Is there any medication you can take to prevent them? [ANSWER]
Esophageal bleeding in PBC usually relates to varices (varicose veins in the esophagus), that can occur as the disease progresses. Once you've bled once then yes you are more likely to bleed again. However for the majority of people bleeding is prevented in advance of any problems: by having gastroscopies routinely, when there are signs on the blood tests or ultrasounds that you may be at risk, varices can be detected before causing you any problems. If varices are seen you can reduce the chance they bleed by taking a beta blocker to lower your blood pressure. These tablets don't stop varices appearing, but do significantly reduce the chance they will cause you problems.
—Dr. Gideon Hirschfield, 2008
Is carbonated water bad for the liver? [ANSWER]
Not to my knowledge!
—Dr. Gideon Hirschfield, 2008
Since there has been much research about Vitamin D, I would like to know if there is a particular dose of Vitamin D that would help PBC ? [ANSWER]
Most clinicians give Vitamin D to patients with PBC to ensure they have adequate levels to protect their bones. We recommend 1000iu daily, and this is usually given with calcium supplements. There are suggestions that Vitamin D supplements may have other benefits for your immune system, but as yet there is no evidence to support this.
—Dr. Gideon Hirschfield, 2008
I recently had a bout of shingles. Is there any connection to my PBC stage 1? My doctor said that it might have been caused by my being 'run down'. Could the fatigue caused by PBC be a factor? [ANSWER]
Shingles is a very common problem, as the virus that causes it can hide in the nerve cells for a long time. Early stage PBC (stage 1) is not associated with shingles. However it is true to say that some people observe that being generally run down can seem to associate with an attack of shingles. That being said lots of patients with PBC have fatigue, but there is no proven link between this fatigue and shingles recurrence.
—Dr. Gideon Hirschfield, 2008
What are some of the reasons why a patient might not respond to URSO? Is oral cortisone an appropriate supplement or substitute ? [ANSWER]
This is a very good question, and we don't know the answer, assuming they are taking the correct dose (13-15mg/kg), and taking it regularly. The earlier you are diagnosed with PBC the more likely you are to gain benefit from Urso. Furthermore although only perhaps half of patients get the maximum response to Urso we still recommend Urso is continued in everyone, as the studies suggest a benefit overall. For those that don't respond there aren't any proven treatments at present. There are trials however under way. Steroids (cortisone) aren't usually used in PBC, because the side effects particularly on the bones, are a problem when it is taken long term.
—Dr. Gideon Hirschfield, 2008
How often should we have a bone scan, if the last one was normal? How often should we have a colonoscopy (colon cancer in family) and what is the indication for having an upper gastro-intestinal endoscopy? [ANSWER]
Bone density measurements are useful because they indicate the risk of a fracture occurring from osteoporosis. However the frequency of repeat bone density measurements once you've got a baseline is not clearly defined, and takes into account your age, your medication, your history of fractures in yourself or your family, and your stage of liver disease. So if someone has very early PBC, and is otherwise well, then if the baseline bone density scan is normal, there may be no need to repeat it at all. Alternatively there may be reasons why your Physician wants a repeat scan ever few years e.g. steroid use, low bone density to start with etc. Colonoscopy is different and the indications for this test continue to change. In terms of screening many people think that everyone over the age of 50 should have one, but this is a major undertaking for the health service! Certainly if there is a strong family history of cancer of the bowel or polyps, most people recommend a look inside. When to repeat the colonoscopy depends on what is found. It may be as soon as 2-3 years or as long as 5-10 years. It's too complicated to be precise for any individual, and your GI specialist can help with the individual details. Gastroscopies are normally performed if there is a significant chance of finding varices in the esophagus. Some physicians will ensure everyone with cirrhosis gets a gastroscopy, whilst others will only do one based on blood tests and ultrasound results (looking for early signs of so called portal hypertension). Again when to repeat a gastroscopy is very dependent on your stage of liver disease, its response to treatment, and what is seen at the first gastroscopy. For some they need a repeat in one year, for others there may never be a another need to look down.
—Dr. Gideon Hirschfield, 2008
Are they recommending the H1N1 flu shot for PBC patients? Are PBC patients at higher risk than the general population? [ANSWER]
All patients with cirrhosis are at risk of flu and in fact are probably considered a high risk group. We all got our flu shots and certainly see no reason from a liver perspective for our patients not to get theirs!
—Dr. Gideon Hirschfield, 2009
Why do some PBC patients develop wheat intolerance? [ANSWER]
True wheat intolerance is an autoimmune disease called celiac disease. It is common in North America (1%) and even more common in PBC patients (6%). This is because it turns out that the genes predisposing to one autoimmune disease are often shared by other autoimmune disease. That is if you have one autoimmune disease, you are at risk of another.
—Dr. Gideon Hirschfield, 2009
What arthritic medication is best tolerated by PBC patients? [ANSWER]
The safest medicine is Tylenol. However Advil like drugs can be used with caution. The only time we worry about Advil drugs is in patients with advanced cirrhosis.
—Dr. Gideon Hirschfield, 2009
I have PBC, I also have a very stubborn sinus infection, are there any antibiotics that will treat the infection but cause minimum damage to my liver? [ANSWER]
Side effects of drugs are not related to having liver disease. You should take the best antibiotics as recommended by a specialist.
—Dr. Gideon Hirschfield, 2009
I have had PBC for approx. 8 years now. Have started taking Omega-3 vitamin pills. Is this safe or even desirable for me to be taking? [ANSWER]
Omega-3 seems safe to our knowledge. Some people think it helps fatty liver but I don't know about PBC. Nothing suggests it will be bad though!
—Dr. Gideon Hirschfield, 2009
Dr. Jenny Heathcote
Dr. Jenny Heathcote graduated from the Royal Free Hospital School of Medicine, London, UK in 1968. After completing her internship and her residency in internal medicine, she was awarded a MRC research fellowship with the late Dame Professor Sheila Sherlock. Her thesis on the transmission of hepatitis B was awarded an MD in 1976.
Dr. Heathcote moved to Stanford, USA, for further research training and joined the Toronto Western Hospital, Canada, 27 years ago, where she has built up an internationally recognized liver research unit, with a major interest in viral hepatitis and autoimmune liver disease. She has been a Professor at the University of Toronto since 1995. She won the Department of Medicine Clinician Teacher award the same year and was given the May Cohen award by the Canadian Medical Association for her mentoring of trainees in 2003.
Dr. Heathcote is a recipient of the Queen’s Jubilee medal for her service to hepatology and received the Canadian Liver Foundation Gold Medal at the Canadian Digestive Diseases Week in 2004. In that year, she also received the Canadian Liver Foundation Lifetime Achievement Award. She has been funded by the Canadian Institutes of Health Research for the last 18 years, and has published over 200 papers in the area of autoimmune liver disease and chronic viral hepatitis.
Dr. Andrew Mason
Dr. Andrew Mason MBBS MRCPI
Professor, Department of Medicine, University of Alberta
Senior Scholar, Alberta Heritage Foundation for Medical Research
Director of Research, Division of Gastroenterology
Director, The Applied Genomics Center
Dr. Mason trained at the Liver Unit, Kings College Hospital, in London, England and then moved to Washington University, St. Louis as a Gastroenterology Fellow to train in molecular virology. At Ochsner Clinic Foundation, New Orleans he became the Medical Director of Liver Transplantation and an Assistant Professor in the Department of Medicine, Tulane University Medical Center. After relocating to the University of Alberta in 2002, Dr. Mason obtained the Scholar award at the Alberta Heritage Foundation for Medical Research Scholar and then became a Senior Scholar in 2008.
Dr. Nigel Girgrah
Dr. Nigel Girgrah received both his MD and PhD at the University of Toronto. He has specialist certification in both gastroenterology and internal medicine with the Royal College of Physicians and Surgeons of Canada. He completed further research training in Hepatology in the area of portal hypertension and his clinical practice and area of research are mainly focuses on end-stage liver disease and transplant hepatology. He is currently the Director of Education for the Multi-Organ Transplant Program at the University Health Network.
Dr. Mark G. Swain
Dr. Mark G. Swain: MD, Queen's University; MSc, Queen's University; FRCPC. Professor, Department of Medicine, University of Calgary, Acting Head, Division of Gastroenterology Immunology and Gastrointestinal Research Group, AHFMR Scholar
Dr. Gideon Hirschfield
Dr. Gideon Hirschfield is a Gastroenterologist and Hepatologist,educated at Oxford and Cambridge, and trained in the United Kingdom. He specialized in General and Transplant Hepatology, and managed an autoimmune liver disease clinic there. He moved to Canada in 2007 to work with Dr. Heathcote at the Liver Clinic in Toronto. His research interests are the genetic basis of liver disease, in particular Primary Biliary Cirrhosis, for which he is heavily involved in the present genome wide study. He is also developing research projects in Autoimmune Hepatitis and Sclerosing Cholangitis.
